Abstract：
Pemphigus is a life-threatening autoimmune blistering disease. Patient characteristics, treatment courses, and outcomes remain unclear owing to its rarity. To describe the background, treatment, and outcomes of pemphigus, we identified 2598 patients with pemphigus vulgaris and 1186 patients with pemphigus foliaceus from a nationwide inpatient database in Japan. Patients with pemphigus vulgaris were younger (62 vs 72 years, P < 0.001), had fewer comorbidities, and were more likely to be admitted to high-volume hospitals (38% vs 30%, P < 0.001) than those with pemphigus foliaceus. Patients with pemphigus vulgaris had undergone more aggressive treatment, including steroid pulse therapy, intravenous immunoglobulin, or plasmapheresis, compared with those with pemphigus foliaceus (48% vs 42%, P = 0.001); specifically, in patients aged <70 years, the pemphigus vulgaris group was more likely to undergo aggressive treatment than the pemphigus foliaceus group (52% vs 45%), whereas there was no significant difference in patients aged ≥70 years (40% vs 40%). Immunosuppressive agents (30% vs 26%, P = 0.015) and analgesics, including opioids (45% vs 36%, P < 0.001), were used more frequently, whereas topical corticosteroids were used less frequently (32% vs 48%, P < 0.001) in patients with pemphigus vulgaris compared with those with pemphigus foliaceus. In-hospital mortality was lower in patients with pemphigus vulgaris than in those with pemphigus foliaceus (2.2% vs 4.0%, P = 0.002); in the comparison stratified by age, the mortality was equivalent among the two groups (0.6% in patients aged <70 years and 6.1% in those aged ≥70 years). Overall, patients with pemphigus vulgaris had a 10-day longer hospitalization period and higher hospitalization costs than those with pemphigus foliaceus. Our findings provide useful information for understanding the current trends in the management of pemphigus in Japan.
摘要：
天疱疮是一种危及生命的自身免疫性起泡疾病。患者特征,治疗课程,由于其稀有性，结果仍不清楚。为了描述背景，治疗,和天疱疮的结果，我们从日本全国住院患者数据库中确定了2598例寻常型天疱疮患者和1186例天疱疮患者.寻常型天疱疮患者年龄较小（62岁vs72岁，P<0.001)，有较少的合并症，并且更有可能入住高容量医院(38%对30%，P<0.001）。寻常型天疱疮患者接受了更积极的治疗，包括类固醇脉冲疗法,静脉注射免疫球蛋白，或者血浆置换,与天疱疮患者相比(48%对42%，P=0.001)；具体地说，在年龄<70岁的患者中，寻常型天疱疮组比天疱疮组更有可能接受积极治疗（52％vs45％），而年龄≥70岁的患者无显著差异(40%vs40%)。免疫抑制剂（30%vs26%，P=0.015）和镇痛药，包括阿片类药物(45%对36%，P<0.001)，被更频繁地使用，而局部使用皮质类固醇的频率较低（32％vs48％，与寻常型天疱疮患者相比，P<0.001）。寻常型天疱疮患者的住院死亡率低于落叶型天疱疮患者（2.2％vs4.0％，P=0.002)；在按年龄分层的比较中，两组的死亡率相当(<70岁患者为0.6%,≥70岁患者为6.1%).总的来说,寻常型天疱疮患者的住院时间比落叶型天疱疮患者长10天,住院费用更高.我们的发现为了解日本天疱疮管理的当前趋势提供了有用的信息。
